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USMLE Renal > Renal and Bladder Cancers, cysts and benign tumours + Renovascular disease > Flashcards

Renal and Bladder Cancers, cysts and benign tumours + Renovascular disease Flashcards

1
Q

What does autosomal dominant polycystic kidney disease present with?

A
  • Numerous cysts in cortex and medulla
  • Causes bilateral enlarged kidneys - ultimately destroying kidney parenchyma
  • Flank pain
  • Hematuria
  • Hypertension
  • UTI
  • Progressive renal failure in 50% of individuals
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2
Q

What mutations cause Aut Dom Polcystic Kidney Disease?

A
  • PKD1 (85% of cases) - chr 16

- PKD2 (15% of cases) - chr 4

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3
Q

What is Aut Dom Polycystic Kidney Disease associated with (other conditions)?

A
  • Berry aneurysms
  • Mitral valve prolapse
  • Benign hepatic cysts
  • Diverticulosis
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4
Q

What is Aut Dom Polycystic Kidney Disease treated with?

A

If hypertension or proteinuria develops treat with:

- ACEi or ARBs

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5
Q

Aut recessive polycystic kidney disease will have what on imaging?

A

Cystic dillation of CDs

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6
Q

Aut recessive polycystic kidney disease presents with?

A
  • In utero may cause Potter sequence
  • May present in infancy

After neonatal period:

  • Systemic hypertension
  • Progressive renal insufficiency
  • Portal hypertension from hepatic fibrosis
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7
Q

What does Autosomal Dominant tubulointerstitial kidney disease cause?

A
  • Causes tubulointerstitial fibrosis and progressive renal insufficiency w. inability to concentrate urine
  • Poor prognosis
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8
Q

What will Autosomal Dominant tubulointerstitial kidney disease look like?

A

Medullary cysts not visualised usually

- Smaller kidneys on US

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9
Q

What is the difference between simple and complex renal cysts?

A

Simple cysts
- Filled with ultrafiltrate (anechoic on US) - majority of renal masses and are typically asymptomatic

Complex renal cysts:
- May be septated, enhanced, or have solid components on imaging - require follow-up or removal due to possibility of RCC

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10
Q

What part of the nephron do RCC originate from?

A

PCT

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11
Q

What do RCC tumours look like?

A

Golden-yellow due to high lipid content (filled with lipid and carbohydrate)
- Polygonal clear cells

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12
Q

What do RCC cells spread?

A

Invade renal vein (may cause varicocele if L side)

  • > IVC
  • > Hematogenous spread
  • > Metastasize to lung and bone
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13
Q

What may RCC present with?

A
  • Hematuria
  • Palpable mass
  • Secondary polycythemia
  • Flank pain
  • Fever
  • Weight loss
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14
Q

How is RCC treated?

A

Surgery/ablation for localised disease

- Ipilimumab or targeted therapy for metastatic disease (rarely curative)

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15
Q

What is RCC resistant to?

A

Chemo and radiation therapy

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16
Q

What is the classic triad of RCC?

A
  • Flank pain
  • palpable mass
  • Hematuria
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17
Q

What is the most common primary renal malignancy?

A

RCC

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18
Q

What group is RCC most common in?

A

Men 50 - 70

- Increased risk in obese and smokers

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19
Q

What is RCC associated with?

A

Paraneoplastic syndromes PEAR

  • PTHrP
  • Ectopic EPO (polycythemia)
  • ACTH (Cushing)
  • Renin (Hypertension)
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20
Q

What is the most common subtype of RCC?

A

Clear cell

21
Q

What genes is RCC clear cell caused by?

A

Gene deletion on chr 3

- Sporadic or inherited as Von Hippel-Lindau syndrome

22
Q

What does Renal oncocytoma arise from?

A

Collecting ducts

23
Q

What kind of tumor is renal oncocytoma?

24
Q

What will renal oncocytoma reveal on histology?

A

Often resected to exclude malignancy

- Large eosinophilic cells with abundant mitochondria w/o perinuclear clearing (vs chromophobe in RCC)

25
What does renal oncocytoma present with?
- Painless hematuria - Flank pain - Abdo mass Same as RCC
26
What is the most common malignancy of early childhood?
Nephroblastoma (ages 2 - 4)
27
What is nephroblastoma also known as?
Wilms tumor
28
What genes is nephroblastoma associated with?
Loss of function mutations of tumour suppressor genes | - WT1 or WT2 on chr 11
29
What tissue does Wilms tumour / nephroblastoma contain?
Embryonic glomerular structures
30
What may nephroblastoma present with?>
- Large palpable unilateral flank mass Possibly: - hematuria - Hypertension
31
What is urothelial carcinoma of the bladder also called?
Transitional cell carcinoma
32
What is the most common tumour of the urinary tract system?
Transitional cell | - Urothelial carcinoma of bladder
33
Where can transitional (urothelial carcinoma) of bladder occur?
- Renal calyces - Renal pelvis - Ureters - Bladder
34
What does urothelial carcinoma of bladder usually present with?
Painless hematuria (no casts)
35
What is urothelial carcinoma of bladder caused by / associated with?
Pee SAC - Phenacetin (withdrawn pain-relief) - Smoking - Aromatic Amines (found in dyes) - Cyclophosphamide
36
What squamous cell carcinoma of the bladder associated with (risk factors)?
- Schistosoma haematobium - Chronic Cystitis - Smoking - Stones
37
What does sq cell carcinoma of the bladder present with?
Painless hematuria (no casts)
38
What sydromes are associated with nephroblastoma (wi;lms tumour)?
- WAGR complex - Denys-Drash syndrome - Beckwith-Wiedemann syndrome
39
What is WAGR complex/
- Wilms tumor - Aniridia (absence of iris) - Genitourinary malfomrations - range of developmental delays WT1 deletion
40
What is Denys-Drash syndrome?
- Wilms tumor - Diffuse mesangial sclerosis (early onset nephrotic syndrome) - Dysgenesis of gonads (male pseudohermaphroditism) WT1 mutation
41
What is Beckwith-Wiedemann syndrome?
- Wilms tumour - Macroglossia - Organomegaly - Hemihyperplasia - Ompahalocele WT2 mutation
42
What Wilms tumor associated syndromes are asociuated with WT1?
- WAGR complex (WT1 deletion) | - Denys-Drash syndrome WT1 mutation
43
What mutation is Beckwith-Wiedemann syndrome associated with?
WT2 mutation
44
What is the most common cause of secondary hypertension in adults?
Renal artery stenosis
45
What are the 2 main causes of RAS?
- Atherosclerotic plaques (proximal 1/3) - older males/smokers - Fibromuscular dysplasia (distal 2/3s) - young females
46
What will unilateral RAS show?
Affected kidney will atrophy (asymmetric kidney size) | - Renal venous sampling will show increased renin in affected kidney and decreased in unaffected
47
What will happen to patients with bilateral RAS when started on a ACEi?
Sudden rise in creatinine
48
What can RAS present with?
- Severe/refractory HTN - Flash pulmonary edema - Epigastric/flank bruit - may have stenosis in other large vessels

USMLE Renal (11 decks)

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